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Esophageal Atresia and Tracheoesophageal Fistula
TEF is typically a congenital condition but has been rarely reported as an
acquired problem after suppuration of mediastinal nodes or as a result of
iatrogenic injury or a complication following foreign-body ingestion. The more
common congenital TEF is accompanied by atresia of the esophagus in more than
85% of patients and generally presents in the immediate perinatal period with
feeding intolerance and inability to handle secretions. These patients undergo
repair shortly after diagnosis, typically via a right thoracotomy or thoracoscopic
approach.
Approximately 3% of all patients with TEF have an “H-type” fistula, in which
both the trachea and the esophagus are intact and patent but are connected by a
fistula that is frequently in the cervical region or high in the thorax (Fig. 124.2 ).
Because there is no accompanying esophageal atresia, these patients are more
likely to present later in infancy or childhood with symptoms of recurrent
respiratory distress or pulmonary aspiration. The acquired form is usually in the
distal trachea or proximal bronchial tree, and is extremely uncommon.
H-type fistulae are notoriously difficult to diagnose, particularly if the fistula
tract is small. Children generally develop recurrent pulmonary infections with no
obvious source. The characteristic history of choking or gagging with swallowing
that accompanies esophageal atresia with TEF may not be present, but parents
may describe excessive secretions or noisy breathing after feeds.
Contrast esophagram, specifically looking for an H-type TEF is necessary.
Most of these fistulae are quite small in diameter (millimeters) and short (also
less than 1 cm), making radiographic identification difficult. Even when contrast
appears in the tracheobronchial tree, it may be difficult to know whether primary
aspiration of orally administered contrast is responsible. Placing a feeding tube in
the esophagus and injecting contrast while pulling the tube back up from the
lower esophagus under fluoroscopic observation may be helpful. This study is
most accurate when performed with the patient in the prone position. Highresolution CT scans may identify the anatomy, but are less commonly performed
to diagnose such fistulae. Bronchoscopy and esophagoscopy may both be
diagnostic and may be useful at the time of repair if a small catheter or wire can